Cases reported "Sjogren's Syndrome"

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11/39. Osteal complications as first manifestation in a patient with primary sjogren's syndrome and with associated distal tubular acidosis (type 1) and chronic renal insufficiency.

    Renal affection is among the complications associated with the sjogren's syndrome. Tubulo-interstitial nephritis constitutes the most frequent renal lesion and distal tubular acidosis (Type 1) is the most important clinical manifestation of this tubular dysfunction, although the occurrence of chronic renal insufficiency is not an uncommon finding in the presence of distal renal tubular acidosis. osteomalacia is a clinical consequence of tubular acidosis caused by buffering of H in the bone. We present the case of a woman with osteal complication a year before being diagnosed with primary sjogren's syndrome and with distal tubular acidosis and renal insufficiency associated at diagnosis.
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12/39. A case of reactive plasmacytosis mimicking multiple myeloma in a patient with primary sjogren's syndrome.

    Primary sjogren's syndrome (pSS) is a chronic autoimmune disease with well-documented association of lymphoid malignancies during the progress of the disease. Although several types of malignancy and pseudomalignancy have been reported in pSS, low-grade non-Hodgkin's lymphomas are the most frequently observed. Reactive plasmacytosis mimicking myeloma is a very rare condition in association with pSS. We describe a 72-yr-old woman with pSS who presented with hypergammaglobulinemia, and extensive bone marrow and lymph node plasmacytosis, which mimicked multiple myeloma. In this patient, there was an abnormal differentiation of memory B cells to plasma cells in the peripheral blood suggesting underlying pathogenetic mechanism for this condition.
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13/39. Tubulointerstitial macrophage infiltration in a patient with hypokalemic nephropathy and primary sjogren's syndrome.

    We report a case of hypokalemic nephropathy associated with primary sjogren's syndrome (SS). The patient presented with profound and persistent hypokalemia secondary to distal renal tubular acidosis (RTA). A renal biopsy exhibited tubular degeneration, marked interstitial fibrosis and intense macrophage infiltration. hypokalemia has been reported to induce macrophage infiltration in experimental animal models but not in humans. This is the first report of intense tubulointerstitial macrophage infiltration in a patient with hypokalemic nephropathy associated with SS.
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14/39. polymyositis: a manifestation of chronic graft-versus-host disease.

    polymyositis developed in a patient who had had bone marrow transplants for the treatment of acute myeloid leukemia. There was no previous evidence of graft-versus-host disease. polymyositis has previously been reported to be associated with graft-versus-host disease; this article suggests that polymyositis may represent its sole manifestation.
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15/39. Scleroderma, Sjogren-like syndrome, and chronic graft-versus-host disease.

    A patient with acute myelogenous leukemia treated with an allogeneic bone marrow transplant developed acute graft-versus-host disease manifested by severe diarrhea, hepatitis, and a cutaneous eruption. As the graft-versus-host disease progressed to the chronic phase, the patient developed marked cutaneous sclerosis and symptoms of xerophthalmia and xerostomia. biopsy of his indurated skin showed features of both graft-versus-host disease and scleroderma. Results of Schirmer's tests, corneal fluorescent studies, parotid flow-rate testing, and a lip biopsy were consistent with sjogren's syndrome. Possibly, activated lymphocytes may have a role in the pathogenesis of graft-versus-host disease, scleroderma, and sjogren's syndrome.
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16/39. Muscle ultrastructure in a case of mixed connective tissue disease and Sjogren's syndrome.

    A 29-year-old black woman with clinical and serological diagnoses of Sjorgren's Syndrome (SS) and mixed connective tissue disease (MCTD) presented a very important muscular compromise with proximal, bilateral weakness and wasting, an abnormal electromyogram, and elevated muscle enzymes. Ultrastructural study of a muscle biopsy showed different degrees of atrophy and presence of subsarcolemmal loss of sarcomeric arrangement forming regions of contractil material with diverse electron densities. capillaries were proliferative with distortion or occlusion of lumen and thickened basement membranes. An unusual capillary abnormality consisting of thick and convoluted basement membrane intermingled with portions of endothelium and macrophages is described. Mononuclear cell infiltration was formed by lymphocytes, plasma cells, macrophages and scarce mast cells. This work is the first study of the ultrastructural muscle pathology in a patient with diagnoses of MCTD and SS based on clinical findings and the presence of antibodies to RNP, SS-A and SS-B antigens.
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17/39. Lymphoplasmacytoid lymphoma elaborating lambda chain paraprotein with amyloid deposition in sjogren's syndrome.

    A woman presented with painful enlargement of her parotid and submandibular glands. She was shown to have the previously unreported combination of idiopathic thrombocytopenic purpura, sjogren's syndrome, Hashimoto's disease, and myasthenia gravis. parotid gland biopsy and bone marrow examination showed the presence of a rare lymphoplasmacytoid lymphoma. There was amyloid deposition in the parotid glands, gums and on muscle biopsy. Immunohistochemical staining of the parotid lymphoma and amyloid was monotypic for lambda light chains, and there was also a lambda chain paraprotein. It is felt that the lymphoplasmacytic lymphoma was responsible for the light chain amyloidosis.
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18/39. gallium-67 uptake in the salivary glands in chronic graft-versus-host disease after bone marrow transplantation.

    Ga-67 citrate scans were performed in a 17-year-old female patient after bone marrow transplantation for acute lymphoblastic leukemia. Ga-67 accumulated in salivary glands in which chronic graft-versus-host disease (GVH) was demonstrated pathologically. Ga-67 scan may be a sensitive and noninvasive test for detecting and monitoring the Sicca syndrome induced by chronic GVH.
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19/39. A case of sjogren's syndrome with severe anemia due to myelitis.

    An unusual case of sjogren's syndrome presenting with severe anemia as the predominant clinical feature is described. Histological examination of a bone marrow biopsy specimen demonstrated that the patient's anemia was caused by myelitis and vasculitis of the small intraosseous vessels. Our report might stimulate a more thorough investigation of bone marrow in patients with connective tissue diseases and anemia.
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20/39. lymphoma in sjogren's syndrome: urinary monoclonal free light chains as a diagnostic aid and a means of tumour monitoring.

    Non Hodgkins lymphoma (NHL) is reported to be at least 40 times more common in sjogren's syndrome (SS). diagnosis may be difficult as blood and bone marrow haematology can remain normal, with no evidence of a serum paraprotein band or Bence-Jones proteinuria by routine electrophoresis. Using the technique of isoelectric focusing in agarose, followed by immunofixation, monoclonal free light chains can be found in the urine of 44% and 74% respectively of patients with NHL and B cell chronic lymphocytic leukaemia, but not in normal individuals. Three patients, two with typical severe primary SS and the third with several features of SS including abnormal sialography and reduced tear secretion, developed B cell NHL of parotid and/or lung. Using the above method on concentrated urine specimens, monoclonal free light chains of the same class as that demonstrated on the tumour cells were found to be present in each patient's urine. In one patient the level of urinary free light chains was monitored and found to correlate with disease activity. Using similar techniques no monoclonal light chains could be found in the urine from a further 10 cases of primary SS and 18 cases of SS secondary to rheumatoid arthritis, all of whom had no clinical evidence of lymphoma. Screening of SS patients' urine by the method described for monoclonal urinary free light chains may provide an early diagnostic clue to the development of lymphoma and be a means of tumour monitoring.
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